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Total parenteral nutrition fibrosis

A A Mhanni, M Kozenko, J N Hartley, M Deneau, W El-Matary, C Rockman-Greenberg
Gaucher disease (OMIM #230800) is caused by β-glucosidase deficiency and primarily involves the mononuclear phagocyte system (also called Reticuloendothelial System or Macrophage System). The disease is classified into three main phenotypes based on the presence or absence of neurological manifestations: non-neuronopathic (type 1), acute neuronopathic (type 2) and chronic neuronopathic (type 3). Typical manifestations include hepatosplenomegaly, skeletal deformities, hematological abnormalities, interstitial lung fibrosis and neurodegeneration in neuronopathic cases...
March 2016: Molecular Genetics and Metabolism Reports
Maria Hukkinen, Annika Mutanen, Markku Nissinen, Laura Merras-Salmio, Helena Gylling, Mikko P Pakarinen
BACKGROUND: Parenteral plant sterols (PSs) are considered hepatotoxic; however, liver PSs and their associations with liver injury in patients with intestinal failure (IF) have not been reported. MATERIALS AND METHODS: We analyzed liver and serum PS (avenasterol, campesterol, sitosterol, and stigmasterol) concentrations and ratios to cholesterol and their associations with biochemical and histologic liver damage in children with IF during (n = 7) parenteral nutrition (PN) and after weaning off it (n = 9), including vegetable oil-based lipid emulsions...
March 9, 2016: JPEN. Journal of Parenteral and Enteral Nutrition
Sue V Beath, Deirdre A Kelly
When cholestasis occurs in patients receiving total parenteral nutrition, it is the result of many pathogenic pathways converging on the hepatic acinus. The result may be a temporary rise in liver function tests. The resulting fibrosis, portal hypertension, and jaundice are hallmarks of type 3 intestinal-associated liver disease to which children are more susceptible than adults. The key to prevention is in identifying high-risk scenarios, meticulous monitoring, and personalized prescription of parenteral nutrition solutions combined with an active approach in reducing the impact of inflammatory events when they occur by prompt use of antibiotics and line locks...
February 2016: Clinics in Liver Disease
Tamir Miloh
The gut-liver axis involves complex interaction between the intestinal microbiome and the liver parenchyma. Probiotics are live microorganisms that are used in a variety of diseases. With currently only 2 randomized-controlled studies (one with Lactobacillus GG and the other with VSL #3), data are scarce to support the clinical effect of probiotic use in children with nonalcoholic fatty liver disease. There is evidence that probiotics decrease the risk of necrotizing enterocolitis and thereby reduce the prevalence of total parenteral nutrition-induced chronic liver disease...
November 2015: Journal of Clinical Gastroenterology
Annika Mutanen, Jouko Lohi, Päivi Heikkilä, Hannu Jalanko, Mikko P Pakarinen
BACKGROUND & AIMS: The pathogenesis of intestinal failure (IF) associated liver disease (IFALD) is uncertain, we therefore investigated the role of FGF19 and pro-inflammatory cytokines has on this disease state. METHODS: Serum FGF19, IL-6 and, TNF-α were measured in 52 IF patients at median age 6.0 years (IQR 2.2-13) after 10 months (4.1-39) on parenteral nutrition (PN). Thirty-nine patients underwent liver biopsies. RESULTS: In IF patients, FGF19 concentrations were lower and those of IL-6 and TNF-α higher compared to healthy matched controls (p ⩽ 0...
June 2015: Journal of Hepatology
Maria Hukkinen, Laura Merras-Salmio, Taina Sipponen, Annika Mutanen, Risto J Rintala, Heikki Mäkisalo, Mikko P Pakarinen
AIMS: This is a descriptive study aiming to compare outcomes of intestinal rehabilitation surgery among pediatric and adult intestinal failure (IF) patients with either primary intestinal motility disorders or short bowel syndrome (SBS) treated by our nationwide program. METHODS: Medical records of IF patients (n = 31, 71% children) having undergone autologous intestinal reconstructions (AIR) (n = 25), intestinal transplantation (ITx) (n = 5), or being listed for ITx (n = 2) between 1994 and 2014 were reviewed...
February 2015: Scandinavian Journal of Gastroenterology
Ana Pinho, Isabel Pinto, Sandra Sampaio, Pedro Neves
We describe the case of a 42-year-old woman who developed encapsulating peritoneal sclerosis (EPS) after 7 years on peritoneal dialysis, with clinical relief by enterolysis associated to treatment with tamoxifen, corticosteroids and parenteral nutrition in haemodialysis. During the next 7 months, she was also conservatively stabilised for mild hyperparathyroidism until she was admitted with calciphylaxis, associated with massive extraosseous calcification. Despite parathyroidectomy and sodium thiosulfate treatment, which resulted in a successful resolution of calciphylaxis, she died 1 year later due to a gross calcified peritoneum...
November 3, 2014: BMJ Case Reports
S Ganousse-Mazeron, F Lacaille, V Colomb-Jung, C Talbotec, F Ruemmele, F Sauvat, C Chardot, D Canioni, D Jan, Y Revillon, O Goulet
BACKGROUND & AIMS: Chronic intestinal failure (CIF) requires long term parenteral nutrition (PN) and, in some patients, intestinal transplantation (ITx). Indications and timing for ITx remain poorly defined. In the present study we aimed to analyze causes and outcome of children with CIF. METHODS: 118 consecutive patients referred to our institution were assessed by a multidisciplinary team and four different categories were defined retrospectively based on their clinical course: Group 1: patients with reversible intestinal failure; group 2: patients unsuitable for ITx, group 3: patients listed for ITx; group 4: patients stable under PN...
June 2015: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
C Lux, J Bergenthal, M Friedl, G Nickenig, D Skowasch
No abstract text is available yet for this article.
May 2014: Deutsche Medizinische Wochenschrift
Cal S Matsumoto, Stuart S Kaufman, Eddie R Island, Bhaskar Kallakury, Nada A Yazigi, Khalid M Khan, Thomas M Fishbein
OBJECTIVE: To evaluate and compare the biochemical and histologic effect of parenteral fish oil lipid emulsion that is rich in omega-3 polyunsaturated fatty acids (O3FAs), Omegaven (Fresenius Kabi AG, Bad Homburg, Germany) with standard omega-6 polyunsaturated fatty acid (O6FA) parenteral nutrition. STUDY DESIGN: Comparison of hepatic explant pathology and biochemical outcome on pediatric patients with intestinal failure treated with either parental O3FA or O6FA who had received a liver-inclusive intestine transplant...
July 2014: Journal of Pediatrics
Tomohiko Ohno, Yoichi Nishigaki, Tetsuya Yamada, Yuko Wakahara, Hiroyasu Sakai, Kotaro Yoshimura, Masahito Shimizu, Toshio Usui, Masaya Saito, Ichiro Yasuda, Hisashi Tsurumi, Eiichi Tomita, Hisataka Moriwaki
Diseases associated with metabolic syndromes are of major concern in developed countries. Nonalcoholic steatohepatitis (NASH) is one of the manifestations of metabolic syndrome in the liver. Previous studies have shown that NASH is also caused by malnutrition. In the present study, a case of malnutrition-associated NASH in a 66-year-old female with anorexia nervosa is reported. The patient had a body mass index (BMI) of only 11.1 kg/m(2) and serum alanine aminotransferase levels of 1,495 IU/l. Steatohepatitis with fibrosis was confirmed by percutaneous liver needle biopsy...
April 2014: Experimental and Therapeutic Medicine
A Waghray, A Nassar, K Hashimoto, B Eghtesad, F Aucejo, V Krishnamurthi, T D Uso, T Srinivas, E Steiger, K Abu-Elmagd, C Quintini
Encapsulating peritoneal sclerosis (EPS) is a rare but devastating complication of peritoneal dialysis characterized by fibrosis and calcification of the intestine that, in severe cases, can progress to intestinal failure and total parenteral nutrition dependency. Medical and surgical interventions carry a poor prognosis in these patients. We describe a case of a 36-year-old female with end-stage kidney disease and severe EPS not amenable to surgical intervention who underwent a combined intestinal and kidney transplantation...
December 2013: American Journal of Transplantation
Rocío Gallego-Durán, Javier Ampuero, Jorge Funuyet, Manuel Romero-Gómez
The most common causes of steatohepatitis are alcohol intake and metabolic disorders. Several methods based on biochemical determinations (carbohydrate deficient transferrin) and questionnaires (AUDIT, CAGE, MALE) are useful for detecting surreptitious alcohol intake. Although new non-invasive methods are under development, based both on lipidomics (Owl-Liver(®)) and on biochemical determinations and anthropometric parameters (NAFLD Fibrosis score) or imaging methods (DeMILI NASH-MRi(®)), none has been proposed as definitive and the gold standard continues to be liver biopsy...
November 2013: Gastroenterología y Hepatología
Kyung-Deuk Hong, Ji Hea Bae, Yun-Jin Jang, Hee-Yeon Jung, Jang-Hee Cho, Ji-Young Choi, Chan-Duck Kim, Yong-Lim Kim, Sun-Hee Park
BACKGROUND/AIMS: Encapsulating peritoneal sclerosis (EPS) is an often-fatal complication of long-term peritoneal dialysis (PD). We here report the clinical features of EPS in Korean PD patients from a single university center. METHODS: The data were collected retrospectively from 606 PD patients at Kyungpook National University Hospital, between August 2001 and August 2011. The diagnosis of EPS was based on clinical signs and symptoms, and confirmed by radiological findings...
September 2013: Korean Journal of Internal Medicine
J F Heidendael, M M Tabbers, I De Vreede
UNLABELLED: Newborn screening for cystic fibrosis enables early diagnosis and treatment, leading to better outcomes for patients with cystic fibrosis. Although the sensitivity of several screening protocols is high, false negative screening results of the newborn patient still occur, which can lead to a significant delay in diagnosis when the awareness for presenting symptoms of cystic fibrosis declines. Neonatal cholestasis is one of the presenting symptoms of cystic fibrosis but can be easily missed when total parenteral nutrition has been given...
December 2014: European Journal of Pediatrics
Hanine Inaty, Maher Tabba
SESSION TYPE: Pleural Student/Resident Case Report PostersPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PMINTRODUCTION: Chylothorax is caused by accumulation of lymphatic fluid (chyle) into the pleural space1,2,3. Trauma and malignancies are the most common etiologies. Only three cases of chylothorax associated with prostate cancer have been reported in the literature1. Here we are presenting another case of chylothorax due to metastatic prostate carcinoma.CASE PRESENTATION: 69 years old male with known prostate cancer and undergoing hormonal therapy, presented with progressive dyspnea...
October 1, 2012: Chest
Neha Kwatra, Eglal Shalaby-Rana, Srikala Narayanan, Parvathi Mohan, Sunil Ghelani, Massoud Majd
BACKGROUND: Hepatobiliary scintigraphy is highly sensitive for diagnosing biliary atresia; however, its specificity has varied in the literature from 35% to 97%. OBJECTIVE: The purpose of this study was to re-evaluate the accuracy of phenobarbital-enhanced hepatobiliary scintigraphy in differentiating biliary atresia from other causes of neonatal cholestasis. MATERIALS AND METHODS: We retrospectively reviewed all hepatobiliary scans of infants with cholestasis at our institution from December 1990 to May 2011...
October 2013: Pediatric Radiology
Robert Spence, Scott Gillespie, Maurice Loughrey, Keith Gardiner
TITLE: Encapsulating peritoneal sclerosis - A 5 year experience. AIM: Encapsulating peritoneal sclerosis (EPS) is a rare, life-threatening condition, characterised by a progressive, intra-abdominal inflammatory process resulting in fibrotic visceral constriction. We report the aetiology, management, and outcome of EPS in Belfast. METHOD: All patients diagnosed with EPS in Belfast over the past 5 years are included. Presentation, aetiology, imaging, pathology, and outcome are retrospectively analysed and reported...
January 2013: Ulster Medical Journal
A Chibishev, Z Pereska, N Simonovska, V Chibisheva, M Glasnovic, L T Chitkushev
INTRODUCTION: In this study, we assess the effectiveness of a conservative therapeutic treatment of acute corrosive poisonings in adults, and we define therapeutic protocols based on clinical and endoscopic criteria. METHODS: We analyzed clinical records of patients with acute corrosive poisonings who were hospitalized and treated at the Toxicology Clinic at the University of Skopje, Republic of Macedonia, during a 5-year period (2006-2010). A total of 481 patients' records with cases of acute corrosive poisonings were analyzed...
June 2013: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
Akio Kubota, Narutaka Mochizuki, Jun Shiraishi, Masahiro Nakayama, Hisayoshi Kawahara, Akihiro Yoneda, Yuko Tazuke, Taro Goda, Kengo Nakahata, Hiroyuki Sano, Shinya Hirano, Hiroyuki Kitajima
BACKGROUND: Parenteral nutrition (PN)-associated liver dysfunction (PNALD) in term infants usually manifests as intrahepatic cholestasis, which recovers with enteral nutrition (EN) in most cases; however, as the number of extremely low-birthweight infants (ELBWI) has been increasing, and consequently intestinal diseases associated with ELBWI have been increasing, more intractable PNALD has been encountered after surgical treatment in ELBWI, which does not resolve or rather worsens with EN...
February 2013: Pediatrics International: Official Journal of the Japan Pediatric Society
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